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对3名患有因子VIII抑制剂的血友病患者进行长期高剂量因子VIII治疗。

Long-term high dose factor VIII treatment of 3 haemophiliacs with factor VIII inhibitor.

作者信息

Scheibel E, Feddersen C, Hertz H

出版信息

Scand J Haematol. 1985 May;34(5):378-84. doi: 10.1111/j.1600-0609.1985.tb00765.x.

Abstract

3 patients with haemophilia A and inhibitor against Factor VIII were developing progressive haemophiliac arthropathy due to the non-feasibility of prophylactic treatment. In order to suppress inhibitor formation, long-term treatment with high-dose Factor VIII (100 units per kg body weight twice daily) was initiated. Prothrombin complex concentrate was given only for bleeding episodes. Though all 3 patients were high responders, they presented different treatment courses. 2 became low responders after 4 and 11 months' treatment, respectively. 1 patient had no demonstrable inhibitor after start of treatment. In all 3 patients, prophylactic treatment was established, in 1 case still with increased doses compared to non-inhibitor patients. The high-dose Factor VIII treatment makes in possible to provide prophylactic treatment for the high-responder inhibitor patients. However, the extremely high costs represent a serious obstacle to this treatment.

摘要

3例甲型血友病且对凝血因子VIII产生抑制物的患者,由于预防性治疗不可行,正在发展为进行性血友病性关节病。为抑制抑制物形成,开始采用高剂量凝血因子VIII(每公斤体重100单位,每日两次)进行长期治疗。仅在出血发作时给予凝血酶原复合物浓缩剂。尽管所有3例患者都是高反应者,但他们呈现出不同的治疗过程。2例患者分别在治疗4个月和11个月后成为低反应者。1例患者在开始治疗后未检测到抑制物。在所有3例患者中,均确立了预防性治疗,其中1例与无抑制物的患者相比,剂量仍有所增加。高剂量凝血因子VIII治疗使得为高反应性抑制物患者提供预防性治疗成为可能。然而,极高的费用是这种治疗的严重障碍。

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